Progress in cardiology
Prevalence of congenital heart disease

https://doi.org/10.1016/j.ahj.2003.05.003Get rights and content

Abstract

Background

Today most patients with congenital heart disease survive childhood to be cared for by adult cardiologists. The number of physicians that should be trained to manage these lesions is unknown because we do not know the number of patients.

Methods

To answer this question, the expected numbers of infants with each major type of congenital heart defect born in each 5-year period since 1940 were estimated from birth rates and incidence. The numbers expected to survive with or without treatment were estimated from data on natural history and the results of treatment. Finally, lesions were categorized as simple, moderate, or complex, based on the amount of expertise in management needed for optimal patient care.

Results

From 1940 to 2002, about 1 million patients with simple lesions, and half that number each with moderate and complex lesions, were born in the United States. If all were treated, there would be 750,000 survivors with simple lesions, 400,000 with moderate lesions, and 180,000 with complex lesions; in addition, there would be 3,000,000 subjects alive with bicuspid aortic valves. Without treatment, the survival in each group would be 400,000, 220,000, and 30,000, respectively. The actual numbers surviving will be between these 2 sets of estimates.

Conclusions

Survival of patients with congenital heart disease, treated or untreated, is expected to produce large numbers of adults with congenital disease, and it is likely that many more adult cardiologists will need to be trained to manage moderate and complex congenital lesions.

Section snippets

Natural history and survival

The tables below attempt to estimate the survival of untreated and treated subjects with the different lesions. The tables start from 1940 because very few subjects with these lesions born before this date will still be alive. The single exception is BAV, in which the peak age for surgery is from 60 to 80 years, so that in this table alone the starting date is 1920. One assumption applied to all the tables is that the incidences of each form of CHD remained constant over the years.

Conclusion

The data presented in Figure 1, Figure 2, Figure 3, Figure 4 are summarized in Figures 5, AD, by adding separately the numbers for simple, moderate, and complex lesions, as well as repeating the bicuspid valve data from Figure 3, D. The simple lesions include VSDs, treated or small PDA, mild or treated PS, and treated total anomalous pulmonary venous connection. In total, 1.2 million children have been born with these lesions since 1940, about the same as the total of the moderate (ASD,

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    Supported in part by Program Project Grant HL 25847 from the National Heart, Lung and Blood Institute, National Institutes of Health.

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